S16 The influence of the CFTR modulator ivacaftor on aspergillosis in cystic fibrosis

IntroductionCystic fibrosis (CF) is a life limiting genetic condition which occurs due to mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Absence of functional CFTR protein leads to progressive respiratory disease characterized by bronchiectasis and chronic infections. CF lung disease predisposes patients to infection and sensitivity to the fungal pathogen Aspergillus fumigatus. Novel CFTR modulating therapies have recently been associated with potential disease modification in CF. It is unclear whether these therapies will have an influence on susceptibility to Aspergillus related disease in CF.MethodsWe conducted a retrospective cohort study examining patients who commenced the CFTR modulator ivacaftor. Over a period of 5 years we monitored the isolation ofAspergillus in sputum samples and patients’ serological response to Aspergillus fumigatus.ResultsIn 40 patients, ivacaftor therapy resulted in a significant decrease in sweat chloride (from 112 [102.75 – 119.25] to 45 [37 – 61], p